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   Table of Contents - Current issue
Coverpage
January-June 2020
Volume 7 | Issue 1
Page Nos. 1-67

Online since Monday, January 20, 2020

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STATE OF THE ART  

Treatment of maxillary hypoplasia in cleft lip and palate patients: Keeping the total burden of care as low as possible p. 1
Maria Costanza Meazzini
DOI:10.4103/jclpca.jclpca_25_19  
Introduction: Maxillary hypoplasia is a common problem in cleft patients. The literature has shown that early treatment is unstable; nevertheless, early maxillary protraction may still be taken into consideration, if specific functional problems exist. On the other hand, long-term results of the application of the Alternate Rapid Maxillary Expansion and Constriction (Alt-RAMEC) technique, a maxillary protraction technique applied closer to the peak of growth, have been shown to be stable. Therefore, a later approach might be considered if the objective is purely occlusal/esthetic. Objective: The objective of this study was to describe the rationale behind a protocol for the treatment of maxillary hypoplasia in cleft lip and palate (CLP) patients where timing is guided by the different indications. Materials and Methods: We subdivided this study into two groups. (1) Early treatment group (preliminary study): 30 unilateral CLP (UCLP) and bilateral CLP patients were treated early with expansion and face mask with two specific functional indications: 22 were affected by middle ear infection, otitis media (OM), with subsequent hearing impairment, and 8 by obstructive sleep apnea (OSAS). The objective of early treatment thus, was purely functional. (2) Late adolescence treatment group (follow-up study): a different group of older patients was treated at a later stage with an esthetic-occlusal aim. Thirty-nine UCLP patients were consecutively treated with the Alt-RAMEC technique at an average age of 13.2 years (11.3–14.2 years) before protraction. The average age at long-term follow-up was 18.3 years (17.4–24.7 years). Results: (1) Early treatment group (preliminary results): the sagittal advancement of A-point, after the application of the face mask and expander, was 2.6 ± 1.2 mm. About 65% of the OM patients had a net improvement of all audiological symptoms (audiometric tests and tympanograms), and 100% of OSAS patients had a significant improvement of obstructive parameters (apnea-hypopnea index). (2) Late treatment group (follow-up study): the sagittal advancement of A-point, after the application of the Alt-RAMEC technique, was 5.6 ± 2.7 mm. The position of the maxilla was stable in the long term. Conclusion: Our results suggest that, even though skeletal relapse is expected after early correction of maxillary hypoplasia, there might be an indication in patients with functional problems, such as hearing or breathing disorders, to avoid more complex treatments. On the other hand, to correct the occlusal esthetic problem related to maxillary hypoplasia, it is advisable to wait till after growth peak and apply a modified Alt-RAMEC technique, with a double-hinged expander and to obtain more stable long-term results. This technique seems to significantly reduce, especially in female patients, the need for final orthognathic surgery.
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REVIEW ARTICLE Top

Approach to patients with cleft lip and palate in orthodontics Highly accessed article p. 8
Yasin Akbulut
DOI:10.4103/jclpca.jclpca_23_19  
Cleft lip and palate (CLP) are the most commonly observed congenital anomaly in the craniofacial region, which are observed in one in every one thousand children and caused by multifactorial factors. CLP occur due to the inability of embryonic protrusions, which forms the roof of the mouth with the maxillary bone, to integrate completely. Due to the scar tissue formed as a result of the operation conducted for dental problems, such as missing teeth or extra teeth, location anomalies, enamel defects, and anomalies of size, formation, and eruption, CLP can cause developmental problems, problems in speech due to difficulties in phonation, and hearing problems due to frequently experienced otitis media. CLP treatment requires a multidisciplinary approach. For the CLP treatment to succeed, it is very important for a team that consists of an orthodontic specialist, ENT specialist, audiologist, psychologist, dentist, and speech therapist to work in harmony. The treatment process of CLP starts as soon as the infant is born. The period of the first 6 months is the preoperative period. In this period, a feeding plaque implementation is conducted by an orthodontic specialist. Operations for lips are usually conducted between the 3rd and 6th months, whereas operations for palates are usually conducted between the 12th and 18th months. In mixed dentition, maxillary expansion and protraction, tooth location correction, and bone grafting are conducted. In continuous dentition, if constant orthodontic treatment is necessary, orthognathic surgical operations and prosthetic dentistry treatments are conducted. In CLP anomalies, the main aim is to educate future mothers and fathers about the factors that can be eliminated. For infants who are born with CLP, the treatment should be initiated without losing any time. The fact that the treatment of these patients is conducted at an esthetically and functionally satisfactory level will also improve the quality of life for both the patients and their families. Furthermore, in patients with CLP, esthetics, phonation, and speech, briefly, the external appearance should be emphasized with recurring operations and follow-ups and their mental states should not be ignored in addition to supporting them with psychological rehabilitation and a constant boost of morale.
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ORIGINAL ARTICLES Top

Reconceiving the closed approach in unilateral cleft lip rhinoplasty: An objective evaluation of 64 consecutive cases p. 17
Chirayu Parwal, Lalit Choudhary, Anurag Pandey, Shiv Shankar Saha, Vivek Kumar
DOI:10.4103/jclpca.jclpca_20_19  
Introduction: The cleft lip nose has been the classical case of an unsolved mystery in the history of plastic surgery. As Kernahan aptly stated, “a repaired cleft is revealed more by the associated nasal deformity than by the repair line.” Cleft lip nasal deformity becomes a surgical challenge due to various reasons: 1. Wide variation in clinical presentation, requiring a host of surgical techniques. 2. Deformity may be quite asymmetric, making correction difficult. 3. Patients might have undergone several previous operations, which lead to scarring. Aim: We aim to assess the surgical outcome of the closed approach of unilateral cleft lip nose deformity correction. Methodology: We perform the cleft lip nose correction with the primary cleft repair as well as a secondary rhinoplasty in preschool age group and adults depending on the esthetic concerns. Our approach is the closed (endonasal) rhinoplasty technique to dissect the slumped alar cartilage of its soft-tissue attachments and cutaneous skin flap and reshape and reposition it with the help of sutures inserted percutaneously. Results: We have evaluated our results by comparing the pre- and post-operative photographs in standard views and represented the correction of several esthetic nasal parameters with a simple numerical rating. We have compiled the overall result by taking into account the collective numerical ratings at 6 months of follow-up. Our results of primary rhinoplasty ranked in the “good” or “excellent” category in 85% of the cases while it was “fair” or “good” in 82% of the secondary rhinoplasties. Conclusion: We conclude that the closed rhinoplasty is less invasive, takes less surgical time, avoids tedious dissection, gives better on table predictability of the outcome, and shortens postoperative recovery and tissue healing, providing a stable and acceptable result.
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Maternal stressful events and socioeconomic status among orofacial cleft families: A hospital-based study p. 24
Moe Myat Noe Phyu, Zayar Lin, Kyaw Myo Tun, Thi Ha Myint Wei, Ko Ko Maung
DOI:10.4103/jclpca.jclpca_19_19  
Background and Objective: Studies have suggested that maternal stressful events are associated with an increased risk of orofacial cleft (OFC) in many populations, but none have focused on Myanmar. Socioeconomic status (SES) has been recognized as a strong predictor of pregnant mothers' perceived maternal stress. We sought to assess the SES among OFC families and determine the role of maternal stressful events during pregnancy. Materials and Methods: A cross-sectional descriptive study was performed at the New Look New Life Cleft Lip and Palate Center/Smile Train Myanmar in the Shwe Baho and Pinlon Hospital in Yangon, Myanmar, from December 2016 to December 2017. The study included 298 mothers of children under-five with OFCs. The research tool included a questionnaire used in face-to-face interviews with mothers. SES was measured with Kuppuswamy's scale. Maternal stress questions from the Kaiser Permanente/California Department of Health Study of Pregnancy and Stress were used. Results: Among the 298 families, 126 (42.28%) household heads were farmers, and the most common educational level was middle school (83, 27.85%). Two-thirds of the respondents reported a monthly family income below 180,000 Kyats (120 USD). Most OFC families (129, 43.3%) had upper lower SES. One hundred and forty-one (47%) mothers reported stressful events during pregnancy, and 106 (60%) of these mothers indicated financial-related burden as the leading cause of stress. SES was significantly related to maternal stress (P = 0.01). Conclusion: Most OFC families had low SES, which could lead to financial-related stress for pregnant mothers. Future studies need to extend the results to better understand this relationship.
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Comparison of maxillofacial growth characteristics in patients with and without cleft lip and palate p. 30
Rohit Khanna, Tripti Tikku, Sneh Lata Verma, Geeta Verma, Saumil Dwivedi
DOI:10.4103/jclpca.jclpca_22_19  
Introduction: The neurotropic growth precedes the somatic growth, in the craniofacial region, any lag or disturbance in the development of cervical vertebrae may attribute to the development of craniofacial cleft. Objective: The aim of this study is to evaluate the developmental relationship of cervical vertebrae and maxillofacial morphology in complete cleft lip and palate patients (untreated and surgically treated) and noncleft patients based on the parameters on lateral cephalograms. Materials and Methods: One hundred and sixty patients were first divided into two groups, Group I patients (6–12 years) and Group II patients (12–18 years). These groups were further subdivided into three subgroups, i.e., noncleft or normal patients, untreated cleft group, and surgically treated cleft group patients. Thirteen angular and 19 linear parameters taken on digital lateral cephalogram were analyzed and discussed to evaluate and compare the growth status and craniofacial morphology. Results: There was no significant delay in skeletal maturity noted in cleft patients when they were compared with their normal counterparts. Cranial base length and the cranial base angle were insignificantly reduced in cleft groups, whereas the maxilla in surgically treated patients was found to be significantly shorter along with retroposition and clockwise rotation with respect to cranial base. Mandible was shorter in length and posteriorly positioned with respect to cranial base with downward and backward rotation in surgically untreated and treated cleft patients, but the difference was statistically nonsignificant. Marked differences in maxillo-mandibular relationship were seen in surgically treated patients suggestive of the establishment of Class III jaw relation in those patients. Conclusion: The iatrogenic repercussion of surgery on cleft patients has a significant restraining effect on maxilla, mandible, and maxillo-mandibular relationship and morbidity do not play a significant role in delaying skeletal maturity.
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Evaluation of efficacy of a novel extraoral nasoalveolar molding technique with lip taping in cleft lip and palate patients p. 43
AK Shafeeq, Sandeep Shetty, Shafees Koya, Akhter Husain, Katheesa Parveen
DOI:10.4103/jclpca.jclpca_18_19  
Objective: The purpose of the study was to evaluate the efficacy of an extraoral nasal molding device (EONMD) with lip taping in the treatment of patients with cleft lip and palate (CLP). The objectives of the study were to compare and evaluate the dimensions of cleft and alveolus, the morphology of the nostrils, and the width of the cleft lip between pre- and posttherapy. Materials and Methods: The study was conducted on seven patients with CLP which included four unilateral and three bilateral clefts. A novel EONMD along with lip taping was used for approximation of clefts. Extraoral and intraoral measurements were done pre- and post-nasoalveolar molding (NAM) therapy. Intraoral measurements included dimensions of cleft and alveolus, and extraoral measurements included the morphology of the nostrils and the width of cleft lip. Results: Intraorally, there was a reduction in intersegment distance. There was also an increase in the arch width along with the larger and smaller segment length which may be attributed to the growth in both unilateral and bilateral CLP. The measurements of the nose revealed a statistically significant increase in bialar width and increase in the columellar length and columellar width in both unilateral and bilateral CLP. The nostril attained its shape because of the nasal molding in all the cases. The lip measurement showed reductions in the cleft lip width. Conclusions: The study has quantitatively shown that the novel approach of presurgical NAM therapy with an EONMD with lip taping has significant advantages in the treatment of unilateral and bilateral CLP patients.
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Is cleft still a taboo? Beliefs and expectations of parents of cleft lip and palate patients: A study from an institute in North India p. 50
Debarati Chattopadhyay, Anindya Das, Rupinder Deol, Vandana Thakur
DOI:10.4103/jclpca.jclpca_13_19  
Introduction: Cleft lip and palate are a common but significantly disfiguring congenital anomaly affecting children. Besides the multiple problems they pose regarding the child's feeding, speech, and hearing, being visible, they cause a major social stigma. Different degrees of parental guilt and shame are frequently encountered, primarily due to the perceived cause of the birth defect. Aims: This study aims to define parental perceptions associated with a child of cleft lip or palate and their social significance. Materials and Methods: A detailed questionnaire was structured based on a previous study by Weatherly-White (2004). A sample of 64 families was selected by total enumerative sampling technique over a period of one year. Tools were translated into Hindi (local language) and validated. Parents not willing to participate or unable to understand Hindi or English were excluded from the study. The data were collected using questionnaire and semi-structured interviews. Ethical clearance was obtained and consent was taken from parents for participation in the study. Results: Parents of 63 patients were interviewed and the results were tabulated. Conclusion: The study identified many cultural and societal attitudes that deeply affect the way that communities treat children with clefts and other facial deformities. The results interestingly almost mirror the last study similar to this, almost 20 years ago. It is surprising to note the beliefs and perceptions about clefts have remained largely unchanged.
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CASE REPORTS Top

Septal aplasia with unilateral cleft lip and palate: A new entity or a variation of the binderoid cleft? p. 54
Serena Martin, Chris Hill
DOI:10.4103/jclpca.jclpca_15_19  
Congenital septal abnormalities are rare. Septal agenesis in isolation has been described in the literature with scant reports of similar septal anomalies, all of which have presented in adulthood with a request for reconstruction. Mulliken described a rare subset of children with cleft lip and palate associated with nasolabiomaxillary hypoplasia. Although some of these cases were similar to ours, none of the described cases were associated with septal aplasia. We report two cases of septal aplasia in combination with a right unilateral cleft of the lip and palate in two babies <1 year of age. We discuss insights learned for primary repair of the lip and palate in these patients and include preoperative photographs.
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Dental phenotype of multiple impacted supernumerary teeth in Wiedemann–Steiner syndrome p. 59
Priyanka Verma, Seema Kapoor, Haseena Sait, Sujoy Ghosh, Sunita Gupta
DOI:10.4103/jclpca.jclpca_12_19  
Wiedemann–Steiner syndrome (WSS) is a rare autosomal dominant disorder characterized by short stature, intellectual disability, developmental delay, hypertrichosis cubiti, and dysmorphic facial features (thick eyebrows with a lateral flare, long eyelashes, vertically narrow and downslanting palpebral fissures, and wide nasal bridge). Dental findings reported with this syndrome are premature eruption of dentition, malocclusion, hypodontia, cleft palate, and retrognathia. We report a 12-year-old boy with a heterozygous two-base pair deletion in exon 22 of the KMT2A gene, resulting in a frameshift and premature truncation of the protein 17 amino acids downstream to codon 1974. Our patient exhibited multiple impacted supernumerary teeth which have never been reported earlier. This extends the dental phenotypic spectrum of WSS. To the best of our knowledge, this is the first reported case from India.
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Unilateral sagittal split ramus osteotomy for facial asymmetry by IIG p. 64
Philip Mathew, Rahul V. C. Tiwari, Paul Mathai, Jisha David, Heena Tiwari, Neeraj Bansal
DOI:10.4103/jclpca.jclpca_16_19  
Treating cases of facial asymmetry is always a challenge for oral and maxillofacial surgeons especially when the face has minor deformities and patients expectations are very high. Various non surgical and surgical techniques including orthognathic and orthomorphic surgeries with graftings have kept their milestones for treating such cases. This report presents a similar case of achieving fullness on unilateral side of face to accomplish symmetry which was treated using an interpoistional iliac graft in unilateral saggital split osteotomy to correct the disharmony of face.
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LETTER TO THE EDITOR Top

Management of velopharyngeal insufficiency: The evolution of care and the current state of the art p. 67
R Shah Harsh, B Takwale Anupam, R Thatte Mukund
DOI:10.4103/jclpca.jclpca_24_19  
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